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Structural biology | Diseases
A team from the I2BC publishes in FEBS Journal an exhaustive review of the various mutations affecting the structure of type A lamins, proteins that make up the envelope of the cell nucleus (nuclear lamina), which lead to premature aging in people with the disease (Progeria).
Progeroid laminopathies are characterized by the premature appearance of certain signs of physiological aging in a subset of tissues. They are caused by mutations in genes coding for A-type lamins or lamin binding proteins. Here, scientists from SB2SM/I2BC review how different mutations causing progeroid laminopathies alter protein structure or protein-protein interactions and how these impact on mechanisms that protect cell viability and function. Targeting these molecular alterations in A-type lamins and associated proteins identified through structural biology studies could facilitate the design of therapeutic strategies to treat patients with rare but severe progeroid laminopathies.
Image adapted from : The Cell Nucleus and Aging: Tantalizing Clues and Hopeful Promises. Scaffidi P, Gordon L, Misteli T. PLoS Biology 2005 3(11): e395. https://doi.org/10.1371/journal.pbio.0030395
A Marcelot, HJ Worman, S Zinn-Justin. Protein Structural and Mechanistic Basis of Progeroid Laminopathies, FEBS J. 2020 Aug16 https://pubmed.ncbi.nlm.nih.gov/32799420/
CEA is a French government-funded technological research organisation in four main areas: low-carbon energies, defense and security, information technologies and health technologies. A prominent player in the European Research Area, it is involved in setting up collaborative projects with many partners around the world.